Primary intracranial peripheral primitive neuroectodermal tumor in an adult patient with aphasia: A rare case report

Primary intracranial peripheral primitive neuroectodermal tumors (pPNETs) are extremely rare malignancies that commonly affect children and adolescents. Only 10 cases over the age of 33 have been reported. pPNETs have an aggressive behavior and a high tendency for local recurrence and distant metastasis. Here, we present a case of supratentorial pPNET that affected the left frontoparietal lobe of a 36-year-old female patient. The patient complained of aphasia during the last 2 months. Aphasia is reported for the first time as a result of a pPNET. In T1-weighted MRI, a large mass with mixed isointense to hypointense signals was observed. The tumor was completely removed. Histopathologic examination was indicative of a small round cell tumor. Immunohistochemical analysis showed positivity for CD99. Presence of EWSR1 gene rearrangement confirmed the diagnosis. The patient's aphasia was gradually resolved post-surgery. Six months follow-up showed no evidence of local recurrence or metastasis.